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Description

The main features of this hereditary syndrome is a trias consisting of 1) hyperostosis frontalis interna, 2) adipositas, and 3) virilism and hirsutism.

A peculiar, noninflammatory, and usually benign osteopathy with usually symmetrical thickening of the frontal, parietal, or occipital bones due to deposits on the internal aspects of the squama frontalis. There may be associated menstrual disorders, virilism, hirsutism, mental disorders, fatigue, somnolence, visual disorders, vertigo, tinnitus, obesity, polyphagia, polydipsia, polyuria, loss of sense of smell, decrease in glucose tolerance, convulsions, and involvement of the second, fith, and seventh cranial nerves with hemiplegia and hemiparesis. Age of onset average about 45 years, and the incidence in females is ca 90 percent. Inheritance is autosomal dominant.

These typical lesions of the frontal bone with associated obesity and virilism were first described by Morgagni. Douglas Hunt Stewart in 1928 again emphasised the association with obesity; Ferdinand Morel in 1930 emphasised the accompanying menstrual disturbance, amenorrhea, and impotence; and S. Moore in 1955 delineated the specific nature of the changes that occur in the frontal bone. The term Morgagni-Trias was introduced by Folke Henschen (1881-1977) in 1937, but the term Morgagni-Stewart-Morel syndrome is now commonly used.

We thank Professor Dr. Gerhard H. Müller, Germany, for correcting an error in the original entry.

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